Evaluation of tricuspid regurgitant jet velocity in thalassemia patients with splenectomy

Background: β-thalassemia is an inherited disorder of β-globin biosynthesis. Dysfunction in hemoglobin chain production, ineffective erythropoiesis, and hemolysis occur in β-thalassemia. Pulmonary arterial hypertension (PAH) is increasingly detected in patients with β-thalassemia, and splenectomy which decreases the need for blood transfusion increases the pulmonary artery pressure (PAP). Objectives: This study aimed to assess the PAP in patients with β-thalassemia (male or female and major or intermedia) who had undergone splenectomy. Methods: A total of 137 patients suffering from β-thalassemia were evaluated during the study. All subjects were referred for cardiac evaluation. Clinical history, presence of cardiac symptoms, and previous splenectomy were noted. Standard M-mode, 2D, and Doppler echocardiographic examinations were performed for all subjects. Patients with a tricuspid regurgitant jet velocity (TRV) ≥2.5 m/s were considered at risk for PAH. Results: Average age of the patients was 21.15±6.68 years. No significant difference was observed in the PAP between the 2 groups of thalassemia major and intermedia and also the 2 sex groups. Indeed, 6.6% of the patients had an increased PAP. The significant finding of the study was that the patients who had had splenectomy were significantly at an increased risk of PAH (P = 0.046). Conclusions: The etiology of PAH in thalassemia is multifactorial such as inflammatory mediators. Also, the absence of the spleen plays an important role in developing a high TRV and PAH.